Intravascular large B-cell lymphoma is a rare type of non-Hodgkin’s lymphoma. Its symptoms can vary greatly, making it difficult to diagnose. This type of lymphoma is aggressive, and outlook for people with this disease is typically poor.

Intravascular large B-cell lymphoma (IVLBCL) is a type of non-Hodgkin’s lymphoma. In IVLBCL, lymphoma cells accumulate within blood vessels, which can affect organ function.

IVLBCL is very rare. It’s been estimated to occur in fewer than 0.5 out of one million individuals each year.

Below, we’ll cover more details about IVLBCL, including its symptoms and how it’s diagnosed and treated. Continue reading to discover more.

IVLBCL is a rare subtype of diffuse large B-cell lymphoma. Diffuse large B-cell lymphomas such as IVLBCL are an aggressive type of non-Hodgkin’s lymphoma.

IVLBCL is characterized by lymphoma cells that build up in the small- and medium-sized blood vessels in your body (small veins and arteries). This accumulation of cells can eventually cause blockages within these blood vessels.

Uninterrupted blood flow is essential for the organs and tissues of your body to function. Because of this, the effects of IVLBCL can lead to serious complications in the affected organs.

While IVLBCL can affect any part of your body, the most commonly affected area is the central nervous system (CNS), which includes your brain and spinal cord. Other areas that are commonly involved include your skin and bone marrow.

Can intravascular large B-cell lymphoma (IVLBCL) be cured?

It’s possible for some people with IVLBCL to reach a complete remission. This is when no signs of cancer can be found in the body after treatment. But it’s still possible for IVLBCL to come back after treatment.

The symptoms of IVLBCL can vary greatly between individuals. This can make it challenging to diagnose.

Many people with IVLBCL have what’s called B symptoms of non-Hodgkin’s lymphoma. These include:

There are different variants of IVLBCL. Each variant is associated with a different area of the body, which can affect symptoms as well:

Classic variant

This variant typically involves your CNS. It can cause neurological symptoms and also often skin symptoms. Some examples of neurological symptoms that can happen with the classic variant include:

Hemophagocytic syndrome-associated variant

This variant typically involves your bone marrow, spleen, and liver. This can lead to a variety of effects, including:

Cutaneous variant

Some people with IVLBCL have disease that’s limited to the skin. Skin symptoms can include red skin rash, plaques, or nodules that often affect the torso and lower body. Skin lesions can be accompanied by:

What exactly causes IVLBCL is unknown. Generally speaking, cancers develop due to genetic changes that cause cells to grow and divide uncontrollably. In the case of IVLBCL, immune cells called B cells are affected.

IVLBCL is associated with a variety of genetic changes. Some of these affect molecules called PD-L1 and PD-L2, which normally keep your immune system from attacking your own tissue. This may help IVLBCL escape the immune system.

Because of how rare it is, little is known about who’s at risk for IVLBCL. Most people are diagnosed between the ages of 60 and 70. Both sexes are affected equally, except for in the cutaneous variant, which occurs more often in people assigned female at birth.

Because of its rarity and the variation in symptoms, IVLBCL can be very challenging to diagnose. There’s also no staging system specific to IVLBCL.

Laboratory tests are often an initial part of diagnosis and may include:

Imaging, particularly CT-PET scans, can be helpful in visualizing affected areas. This can also guide a doctor on where to collect a biopsy from. CT scan and MRI scans may also be used during diagnosis.

Biopsy is vital for diagnosing IVLBCL. This may involve one or a combination of deep skin biopsy, bone marrow biopsy, or biopsy from another affected organ.

A doctor can use biopsies to look for an accumulation of lymphoma cells in blood vessels. Testing called immunohistochemistry can be done on biopsy samples to look for cellular markers associated with IVLBCL.

IVLBCL is treated using a combination of drugs called R-CHOP. This includes the following:

R-CHOP doesn’t cross the blood-brain barrier into your CNS, so additional treatment is needed when CNS involvement is confirmed or suspected. This typically involves methotrexate given intrathecally.

IVLBCL is an aggressive type of lymphoma and progresses quickly. Because of this, the outlook for people with this disease is quite poor.

A 2017 study of people with IVLBCL in the United States found that the overall 5-year survival rate for IVLBCL was 46.3%.

Some factors that may lead to a poorer outlook for people with IVLBCL include:

  • older age
  • CNS involvement
  • the hemophagocytic syndrome-associated variant
  • high levels of lactate dehydrogenase

People with the cutaneous variant typically have a better outlook, with research finding an overall 3-year survival rate of 56%. This may be because the presence of skin lesions leads these individuals to seek diagnosis and treatment earlier.

IVLBCL is a rare subtype of diffuse large B-cell lymphoma, an aggressive non-Hodgkin’s lymphoma. It’s characterized by the accumulation of lymphoma cells in small- and medium-sized blood vessels.

The symptoms of IVLBCL vary greatly and can depend on the part of your body that’s affected. This can make IVLBCL very challenging to diagnose.

Because IVLBCL is aggressive, it progresses rapidly, and the outlook of people with this cancer is typically poor. While treatment can lead to complete remissions, IVLBCL often comes back after treatment.